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Red urine. Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy ( > 5cells/ul in fresh uncentrifuged urine, >3 cells/HPF in centrifuged sediment.
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Red urine • Hemoglobinuria • Myoglobinuria • Drugs (rifampicin),food • Inborn errors of metabolism(porphyria) • Urate crystals • Hematuria • Do dipstick,microscopy (>5cells/ul in fresh uncentrifuged urine, >3 cells/HPF in centrifuged sediment.
Red if fresh(bladder), or brown color as Hb converted to acid haematin by urinary acids • Early hematuria:urethral cause • Terminal hematuria:bladder cause • Microscopy:dysmprhic RBC,RBC cast:glomerular bleeding • Prevelance of .5-1.6% in school children
History • Fever,urinary symptoms….infection • Colicky loin pain,lower tract symptoms…..renal calculus • Rash,arthritis…HSP,SLE • Preceding URTI…..PSGN,IgA nephropathy • Coagulopathy,trauma,tumor,exercise • FH of hematuria,deafness,renal failure…Alport • O/E:HTN,kidneys,genitalia,rash
Investigations • Urine protein/creat ratio • EUC,albumin • ASOT,C3,C4,ANA • FBC,clotting studies • Urine calcium/creat ratio • U/S ,XRAY,CT • Urine analysis on parents • IgA,cystoscopy • Renal biobsy
Macroscopic hematuria • Most common cause is infection,then perineal irritation,trauma • Viral infections,adenovirus 11,12 may cause hemorrhagic cystitis • Exercise induced hematuria not associated with renal disease. • Recurrent gross hematuria as IgA nephropathy,Alport,nut cracker (thin,loin pain,compression of renal vein bet aorta,SMA
Causes of hematuria • 1.Glomerular causes • Familial benign hematuria • GN:primary as postinfectious,MPGN,IgA nephropathy • secondary GN as SLE,HSP. • HUS,interstital nephritis,renal vein thrombosis,cystic renal disease • Alport
Non glomerular causes • UTI • Hypercalcuria,renal calculi • Trauma,exercise • Coagulpathy as sickle • Vascular malformations • Nut cracker syndrome • Menarche • Malignancy as nephroblastoma of the kidney or bladder tumors
Persistent MA without proteinuria : • 1.Beningn familial hematuria • 2.idiopathic hypercalcuria • 3.IgA nephropathy • 4.Alport syndrome
Persistent MA without proteinuria : • 1.Beningn familial hematuria • 2.idiopathic hypercalcuria • 3.IgA nephropathy • 4.Alport syndrome
Alport Syndrome • 80% XL,20% AR • Renal failure,sensorineural deafness higher frequencies,ocular changes(anterior lenticonus,retinal changes • Present as micro and rarely macroscopic hematauria with URTI • Proteinura,HTN later age
Diagnosis by EM:Thinning of GBM,split and duplicated lamina densa,basket weave • Males progress to ESRD,deafness by 30y • ACEI may delay progression to ESRD • Defiency of α5 of type 4 collagen
Benign Familial Hematuria(TBMN) • AD inheritance • Present as microscopic hematuria,no proteinuria or renal failure • EM:thinning of GBM • Follow up for proteinuria,HTN
IgA nephropathy • Recurrent macroscopic hematuria,loin pain 1-2 days following URTI,last < 3 days. • Persistent microscopic hematuria ±proteinuria • Nephritic,nephrotic syndrome rare • Present second decade,more in males
Familial cases reported • IgA high in 35-50% • Diagnosis:LM:focal or diffuse mesangial cell proliferation,expansion of mesangial matrix • IM:IgA,C3 deposits • Prognosis for children better than adults • Young children without macroscopic hematuria have the best long term outcome
Heavy proteinuria is a risk factor for progression to ESKD. • Progression to ESRD is slow(25% need dialysis in 20y) • Children with nephrotic syndrome are treated with steriods • ACEI are used to delay progression,decrease proteinuria • Fish oil
PROTEINURIA • DIPSTICK values • .trace (.15g/l),+1 (.3g/l),+2 (1g/l),+3 (3g/l),+4 (20g/l) • False postive when urine concentarted,alkaline urine • Children excrete 150 mg/day of protein or 60-240 mg/m2/day • 40 % of normal urinary protein is tissue rather than plasma as tamm-horsfall (a gp secreted in TALH ) • Almost of the flitered smaller proteinare absorbed in the proximal CT.
Urine protein/creat ratio correlates with measurement of protein in 24 hour collection. • Most normal subjects have a ratio below.02 mg/umole (.2g/g) in above 2y,.05 in children 6-24 m • Micoalbuminuria ranges 30-300 mg albumin/g creat • B2-microglobulin in urine indicates proximal tubular injury
Classification • 1-Functional:seen in stress,fever,cold,exercise,CHF • 2-Idiopathic transient or intermittent • 3-postural:in adolesents,males,less than 2g/day,proteinuria in upright position but not when recumbent • Decrease in amount with age • Has a good outcome
4-pathologic:increase permeability of GBM,loss of anions(nephrotic,GN) • Loss of nephron mass:single kid,cystic disease from hyperfilteration • secretory,overflow:UTI,in newborns as tam